Alexa’s Story - Congenital Diaphragmatic Hernia (CDH)
Prior to Alexa, we always had an image of pregnancy being a certain way and never knew any different. At 30 weeks into our pregnancy, we were given the worst news of our lives and later were given a beautiful miracle. We had a referral to the UofM Maternal-Fetal Medicine Clinic and were told Alexa had a severe congenital anomaly (CDH) with an LHR ratio of 0.83. This was giving her less than a 10% chance to live. We were not offered palliative abortion due to the gestational age (though we personally would not have accepted the offer if given despite how early or far along we were). From that day, our lives changed forever.
As our lives molded around all of the appointments, ultrasounds, and tests, we eventually relocated at 39 weeks to be closer to the city. Three days into our relocation, we welcomed Alexa on 3/17/18 (St. Patrick's Day) at the UofM Masonic Children's Hospital in Minnesota. Immediately after the uncomplicated vaginal delivery, Alexa was intubated and slowly titrated up on supported therapy, from an oscillator, to dual therapy with nitric oxide and eventual progression, to ECMO therapy around 24 hours after birth. Alexa was on ECMO for 12 days and had a sudden significant improvement with her echocardiogram and a lung bud appearing.
The timing of improvement was truly serendipitous. In the preceding days, there was a concern about an overall plateau in progress and a clot/fibrin developing in the arterial cannula of her ECMO tubing. In cases like these, the chance of stroke increases, and discussion of risk/benefit to continue ECMO happens.
The improvement allowed us to trial clamp outs, which Alexa passed and was able to be decannulated.
Decannulation was an array of emotions: happy for such amazing progress but nervous because we were told she would most likely be unable to be placed on ECMO again if she were to decline. There were several days after coming off ECMO until surgery, and we counted and prayed every minute of it would be "boring" or uneventful, without decline or sounding alarms. We think every NICU parent will agree, the noise of a sounding alarm on the monitor, like a low O2 saturation warning, is a sound that haunts our memories forever.
Surgery day was on 4/3/2018 (17 days following delivery). The surgery lasted around 3 hours, with no complications. Postoperatively, we had some setbacks along the way with a pneumothorax, intermittent runs of SVT, and possible infection concerns. However, no matter the obstacle, she continued to progress and was finally discharged home after being in the NICU for 66 days.
The following is a summary of post-discharge to present:
We were discharged home on low flow oxygen with a nasal cannula (no tracheostomy), liquid Zantac, and sildenafil. At home, we lived through the challenge of attempting to get Alexa to feed adequately. When they say feeding would be the most challenging part of post-surgery, we simply didn't believe that could be the case, but it was. We completed outpatient speech and occupational therapies to help with sensory and feeding concerns (from being intubated and in the NICU so long).
Many appointments and much persistence later, Alexa was able to maintain without the need for a G-tube.
She graduated from oxygen therapy around six months of life.
Alexa had a second repair at nine months of age and did well, only staying in the hospital for four days. She came off her sildenafil around 15 months of age and has been free of pulmonary hypertension ever since.
Alexa has needed to be hospitalized a few times due to hypoxia secondary to viral/bacterial respiratory illnesses. She did require oxygen therapy each time but never needed more extensive treatment. She was always able to go home without the need for continued oxygen therapy.
Most recently, Alexa had her third repair since the Gortex patch doesn't grow with her. She did well again and was out of the hospital in 3 days. She is a thriving and active child with no limitations. She gets winded slightly with significant exertion but otherwise acts like other children her age.
We wanted to share Alexa's story because we rode that emotional roller coaster before and after our NICU journey. What's more, we also witnessed a miracle: we were given our daughter, and she has a beautiful quality of life to live. We hope this provides some comfort because few supportive success stories could be found when we started researching CDH. I wish we would have seen this website sooner.
Remember this: babies are resilient, and their tiny bodies can conquer a lot if given the appropriate tools.
Update:
Alexa continues to amaze us. She is growing like a weed (with height). She continues to see Pulmonology, Cardiology, and General Surgery for maintenance monitoring.
She does take a daily inhaler and continues on reflux medication.
Since being on the daily inhalers, we have noticed she has not required hospitalizations when getting a respiratory illness. Whether that be related to her lung maturity, adequate pulmonary management, or both, either way, it’s a win!
We recently met with her General Surgeon for her routine check-up. We hope to get one more year out of her current patch (which was placed two years ago). We have had three surgeries on her diaphragm, with the next one being her fourth.
Alexa is a “spicy,” sweet, fun, determined, and loving little girl. It is so fun to see this personality blossom, knowing that it had a big part of her drive/success in her NICU days. She currently goes to preschool and loves it.
As she has grown, she has had more and more questions about “mommy when I was in the hospital….” Or “daddy, this scar is what I had after I was born?”
We have enjoyed teaching Alexa to be confident in herself and embrace the scars that made her such a hero—allowing her to grow in the questions and curiosity of discovering why she is such a “Tiny Hero” in our eyes. This stands true for all children, but she has a little extra flare in her cape.
Keep the hope!